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Definition
Congenital aural atresia (CAA) is defined as the absence or underdevelopment of the external auditory canal (EAC) and middle ear structures. Occurring once in every 10,000–20,000 live births, CAA represents an uncommon condition affecting males more commonly than females. Unilateral cases are significantly more common than bilateral (in a 7:1 ratio) with the right ear more often affected than the left. Although several syndromes are associated with CAA (including Goldenhar, Treacher Collins, and hemifacial microsomia), 90% of cases are sporadic and 5% appear to be inherited but nonsyndromic (Kesser and Jahrsdoerfer 2010).
While a detailed discussion of embryology of the earis beyond the scope of this entry, it is essential to understand that the middle ear and external ear develop from both first and second branchial arch structures. Since the auricle develops from the six hillocks of His (derivatives of the...
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References
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Hubbard, M.A., Kesser, B.W. (2013). Surgery for Congenital Aural Atresia. In: Kountakis, S.E. (eds) Encyclopedia of Otolaryngology, Head and Neck Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-23499-6_572
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